AB0632 Intracardiac thrombosis and vascular involvement in Behçet's disease : two sides of the same coin ?
Authors: S. Chadli, H. Khibri, W. Ammouri, M. Maamar, H. Harmouche, Z. Tazi Mezalek
Journal: Annals of the Rheumatic Diseases (2022)

Background Behçet’s disease (BD) is a relapsing vasculitis that can affect vessels of all sizes and kinds. Intracardiac thrombosis (ICT) is a serious complication of the disease, which usually presents associated to vascular lesions. However, its mechanism of constitution in BD remains unclear and the link between these manifestations has yet not been fully clarified. Objectives Our first objective is to describe the clinical characteristics, imaging features, therapeutic management and outcome of these patients. And secondly, to shed some light on the relation between this rare condition and the vascular involvement of the disease. Methods This monocentric study was conducted in the University Hospital Center Ibn Sina of Rabat (Morocco), and retrospectively included 312 Behçet patients admitted to the Internal Medicine department between 2010 and 2021. Twelve patients with ICT related to BD were enrolled. Results Patients were mostly male (90%) with a mean age of 30 years. ICT was the presenting symptom of BD in half of the cases. In the other half, it was detected with a mean of 6.4 years after the disease onset. Six patients had a history of deep vein thrombosis (DVT) related to BD, either as a single episode (n=3), recurrent episodes (n=2), or as DVT associated to pulmonary artery occlusion (n=1). The most frequent symptoms were dyspnea (50%), chest pain (50%), dry cough (40%) and hemoptysis (30%). Four patients had signs of right cardiac failure. Peripheral vascular manifestations were noted in five patients. The other Behçet’s flares simultaneously observed were mucocutaneous (80%), digestive (10%), ocular (10%) and articular (10%). Inflammatory parameters were raised in all patients with a median ESR of 49 mm/h and CRP of 51 mg/L. On echocardiography, ICT was always located in the right heart, with an extension to the inferior vena cava in half of the cases. Two patients had also the left cavities involved. Multiple thrombi were visualized in three cases. The associated cardiac lesions observed were pericarditis (50%), tricuspid insufficiency (50%), mitral insufficiency (10%), endomyocardial fibrosis (10%) and myocarditis (10%). The right cavities were dilated among five patients. Pulmonary hypertension was found in 60% of the cases. On CT Chest Angiography, 70% of the patients had pulmonary artery thrombosis and 50% of them had pulmonary artery aneurysms. The other vascular lesions detected were Budd Chiari syndrome with mesenteric ischemia (10%), subtotal internal jugular vein obstruction (10%) and cerebral venous sinus thrombosis (10%). All patients were treated with colchicine, glucocorticoids and immunosuppressants. Eleven patients were put under Cyclophosphamide and one under Methotrexate followed by a TNF- alpha inhibitor. Six patients were also on curative anticoagulation. No death or relapse was recorded to our knowledge. Five patients were declared on complete remission, two on partial remission. Conclusion ICT is a serious complication that usually occurs in the first years of the BD’s onset, among young men with vascular manifestations. We believe that this strong association is highly suggestive that ICT in BD is a direct consequence of the underlying vasculitis. References [1]Wang H, Guo X, Tian Z et al. Intracardiac thrombus in patients with Behcet’s disease: clinical correlates, imaging features, and outcome: a retrospective, single-center experience. Clin Rheumatol. 2016 ; 35(10):2501-7; [2]Getting to the heart of the matter : diagnostic tools and therapeutic approach to cardiac involvement in Behçet syndrome. A Tunisian case series. 2021; 73 (1): 32-43. [3]Emire Seyahi. Behçet's disease: How to diagnose and treat vascular involvement. Best Pract Res Clin Rheumatol. 2016; 30(2):279-295 Disclosure of Interests None declared